Calcium-binding proteins in skeletal muscles of the mdx mice: potential role in the pathogenesis of Duchenne muscular dystrophy

dc.creatorPertille, A
dc.creatorde Carvalho, CLT
dc.creatorMatsumura, CY
dc.creatorNeto, HS
dc.creatorMarques, MJ
dc.date2010
dc.dateFEB
dc.date2014-11-14T20:27:33Z
dc.date2015-11-26T17:16:50Z
dc.date2014-11-14T20:27:33Z
dc.date2015-11-26T17:16:50Z
dc.date.accessioned2018-03-29T00:04:59Z
dc.date.available2018-03-29T00:04:59Z
dc.identifierInternational Journal Of Experimental Pathology. Wiley-blackwell Publishing, Inc, v. 91, n. 1, n. 63, n. 71, 2010.
dc.identifier0959-9673
dc.identifierWOS:000273018200008
dc.identifier10.1111/j.1365-2613.2009.00688.x
dc.identifierhttp://www.repositorio.unicamp.br/jspui/handle/REPOSIP/78343
dc.identifierhttp://www.repositorio.unicamp.br/handle/REPOSIP/78343
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/78343
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1282366
dc.descriptionFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.descriptionP>Duchenne muscular dystrophy is one of the most common hereditary diseases. Abnormal ion handling renders dystrophic muscle fibers more susceptible to necrosis and a rise in intracellular calcium is an important initiating event in dystrophic muscle pathogenesis. In the mdx mice, muscles are affected with different intensities and some muscles are spared. We investigated the levels of the calcium-binding proteins calsequestrin and calmodulin in the non-spared axial (sternomastoid and diaphragm), limb (tibialis anterior and soleus), cardiac and in the spared extraocular muscles (EOM) of control and mdx mice. Immunoblotting analysis showed a significant increase of the proteins in the spared mdx EOM and a significant decrease in the most affected diaphragm. Both proteins were comparable to the cardiac muscle controls. In limb and sternomastoid muscles, calmodulin and calsequestrin were affected differently. These results suggest that differential levels of the calcium-handling proteins may be involved in the pathogenesis of myonecrosis in mdx muscles. Understanding the signaling mechanisms involving Ca2+-calmodulin activation and calsequestrin expression may be a valuable way to develop new therapeutic approaches to the dystrophinopaties.
dc.description91
dc.description1
dc.description63
dc.description71
dc.descriptionFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.descriptionConselho Nacional de Pesquisas [306689/06-5, 301386/07-2, 474708/06-3]
dc.descriptionFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.descriptionFAPESP [95/6110-2, 01/00570-4, 04/15526-9]
dc.descriptionConselho Nacional de Pesquisas [306689/06-5, 301386/07-2, 474708/06-3]
dc.languageen
dc.publisherWiley-blackwell Publishing, Inc
dc.publisherMalden
dc.publisherEUA
dc.relationInternational Journal Of Experimental Pathology
dc.relationInt. J. Exp. Pathol.
dc.rightsfechado
dc.rightshttp://olabout.wiley.com/WileyCDA/Section/id-406071.html
dc.sourceWeb of Science
dc.subjectcalmodulin
dc.subjectcalsequestrin
dc.subjectcardiac muscle
dc.subjectDuchenne muscular dystrophy
dc.subjectextraocular muscles
dc.subjectmdx
dc.subjectmyonecrosis
dc.subjectIntrinsic Laryngeal Muscles
dc.subjectNeuromuscular-junction
dc.subjectExtraocular-muscle
dc.subjectDeficient Cardiomyopathy
dc.subjectDrastic Reduction
dc.subjectGene-expression
dc.subjectMouse Muscle
dc.subjectCalsequestrin
dc.subjectPhenotype
dc.subjectDiaphragm
dc.titleCalcium-binding proteins in skeletal muscles of the mdx mice: potential role in the pathogenesis of Duchenne muscular dystrophy
dc.typeArtículos de revistas


Este ítem pertenece a la siguiente institución